Cone reconstruction for Ebstein's anomaly: Patient outcomes, biventricular function, and cardiopulmonary exercise capacity.

OBJECTIVE: Cone reconstruction is advocated to treat severe tricuspid valve (TV) regurgitation associated with Ebstein's anomaly. Data on postoperative clinical status, ventricular adaptation, and objective cardiopulmonary testing are lacking in these patients.

METHODS: The clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data from 27 consecutive cone reconstructions, undertaken from 2009 to 2013, were retrospectively compared between preoperative baseline and follow-up.

RESULTS: There were no deaths. The cone TV functioned well in all but 1 patient with late dehiscence of inferior annuloplasty sutures that were subsequently repaired. Four patients required pacemaker insertion (3 for new complete heart block). At median follow-up of 2.7 ± 1.5 years, tricuspid regurgitation was reduced in all patients, without causing stenosis. Global left ventricle function remained unchanged (pre-operative fraction 60% ± 4% vs postoperative fraction 61% ± 3%; P = .96). MRI showed enhanced forward pulmonary flow (pre 26 ± 1 mL/beat vs post 36 ± 10 mL/beat; P < .005) and increased left ventricle filling (body surface area-indexed left ventricle end-diastolic volume pre 49 ± 14 mL/m(2) vs post 60 ± 14 mL/m(2); P < .005). New York Heart Association functional class improved (pre 2.5 ± 0.6 vs post 1.3 ± 0.6; P < .0001) and there was significant improvement in peak oxygen uptake (pre 54% ± 18% vs post 66% ± 22%; P = .02).

CONCLUSIONS: Cone reconstruction of TV offers an effective repair in patients with severe regurgitation associated with Ebstein's anomaly. The patients' clinical status improved with better left ventricle filling and objective exercise capacity. The durability of repair, and mechanisms by which the ventricles adapt to the new loading conditions, need longer-term study.